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B-cell lymphoma

MedGen UID:
86953
Concept ID:
C0079731
Neoplastic Process
Synonyms: B-cell lymphomas; B-cell neoplasm
SNOMED CT: B-cell lymphoma (109979007); B-cell lymphoma (clinical) (109979007); B-cell neoplasm (413616009)
 
HPO: HP:0012191
Monarch Initiative: MONDO:0004095

Definition

A type of lymphoma that originates in B-cells. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVB-cell lymphoma

Conditions with this feature

Severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, NK cell-negative, due to adenosine deaminase deficiency
MedGen UID:
354935
Concept ID:
C1863236
Disease or Syndrome
Adenosine deaminase (ADA) deficiency is a systemic purine metabolic disorder that primarily affects lymphocyte development, viability, and function. The clinical phenotypic spectrum includes: Severe combined immunodeficiency disease (SCID), often diagnosed by age six months and usually by age 12 months; Less severe "delayed" onset combined immune deficiency (CID), usually diagnosed between age one and ten years; "Late/adult onset" CID, diagnosed in the second to fourth decades; Benign "partial ADA deficiency" (very low or absent ADA activity in erythrocytes but greater ADA activity in nucleated cells), which is compatible with normal immune function. Infants with typical early-onset ADA-deficient SCID have failure to thrive and opportunistic infections associated with marked depletion of T, B, and NK lymphocytes, and an absence of both humoral and cellular immune function. If immune function is not restored, children with ADA-deficient SCID rarely survive beyond age one to two years. Infections in delayed- and late-onset types (commonly, recurrent otitis, sinusitis, and upper respiratory) may initially be less severe than those in individuals with ADA-deficient SCID; however, by the time of diagnosis these individuals often have chronic pulmonary insufficiency and may have autoimmune phenomena (cytopenias, anti-thyroid antibodies), allergies, and elevated serum concentration of IgE. The longer the disorder goes unrecognized, the more immune function deteriorates and the more likely are chronic sequelae of recurrent infection.
X-linked immunodeficiency with magnesium defect, Epstein-Barr virus infection and neoplasia
MedGen UID:
477076
Concept ID:
C3275445
Disease or Syndrome
XMEN is an X-linked recessive immunodeficiency characterized by CD4 (186940) lymphopenia, severe chronic viral infections, and defective T-lymphocyte activation (Li et al., 2011). Affected individuals have chronic Epstein-Barr virus (EBV) infection and are susceptible to the development of EBV-associated B-cell lymphoproliferative disorders. Magnesium supplementation may be therapeutic (summary by Li et al., 2014).
Lymphoproliferative syndrome 1
MedGen UID:
765548
Concept ID:
C3552634
Disease or Syndrome
Lymphoproliferative syndrome-1 is an autosomal recessive primary immunodeficiency characterized by onset in early childhood of Epstein-Barr virus (EBV)-associated immune dysregulation, manifest as lymphoma, lymphomatoid granulomatosis, hemophagocytic lymphohistiocytosis, Hodgkin disease, and/or hypogammaglobulinemia. Autoimmune disorders, such as autoimmune hemolytic anemia or renal disease, may also occur. Patients show a high EBV viral load and decreased invariant natural killer T cells. It is unknown whether patients with ITK mutations are intrinsically susceptible to development of lymphoma or dysgammaglobulinemia in the absence of EBV infection (summary by Stepensky et al., 2011; Linka et al., 2012). For a discussion of genetic heterogeneity of lymphoproliferative syndrome, see XLP1 (308240).
Leukemia/lymphoma, b-cell, 2
MedGen UID:
854626
Concept ID:
C3887918
Neoplastic Process
Immunodeficiency 36
MedGen UID:
863371
Concept ID:
C4014934
Disease or Syndrome
Immunodeficiency-36 with lymphoproliferation (IMD36) is an autosomal dominant primary immunodeficiency with a highly heterogeneous clinical phenotype, characterized primarily by recurrent respiratory tract infections, lymphoproliferation, and antibody deficiency. Other features include growth retardation, mild neurodevelopmental delay, and autoimmunity. The major complication is development of B-cell lymphoma (Elkaim et al., 2016).
Immunodeficiency 64
MedGen UID:
1684716
Concept ID:
C5231402
Disease or Syndrome
Immunodeficiency-64 with lymphoproliferation (IMD64) is an autosomal recessive primary immunodeficiency characterized by onset of recurrent bacterial, viral, and fungal infections in early childhood. Laboratory studies show variably decreased numbers of T cells, with lesser deficiencies of B and NK cells. There is impaired T-cell proliferation and activation; functional defects in B cells and NK cells may also be observed. Patients have increased susceptibility to EBV infection and may develop lymphoproliferation or EBV-associated lymphoma. Some patients may develop features of autoimmunity (summary by Salzer et al., 2016, Mao et al., 2018, and Winter et al., 2018).
Immunodeficiency 76
MedGen UID:
1781281
Concept ID:
C5543004
Disease or Syndrome
Immunodeficiency-76 (IMD76) is an autosomal recessive primary immunologic disorder characterized by onset of recurrent bacterial, viral, and fungal infections in early childhood. Laboratory studies show T-cell lymphopenia and may show variable B-cell or immunoglobulin abnormalities. More variable features found in some patients include lymphoma and neurologic features. Although bone marrow transplantation may be curative, many patients die in childhood (summary by Lyszkiewicz et al., 2020).
Immunodeficiency 82 with systemic inflammation
MedGen UID:
1781752
Concept ID:
C5543581
Disease or Syndrome
Immunodeficiency-82 with systemic inflammation (IMD82) is a complex autosomal dominant immunologic disorder characterized by recurrent infections with various organisms, as well as noninfectious inflammation manifest as lymphocytic organ infiltration with gastritis, colitis, and lung, liver, CNS, or skin disease. One of the more common features is inflammation of the stomach and bowel. Most patients develop symptoms in infancy or early childhood; the severity is variable. There may be accompanying fever, elevated white blood cell count, decreased B cells, hypogammaglobulinemia, increased C-reactive protein (CRP; 123260), and a generalized hyperinflammatory state. Immunologic workup shows variable B- and T-cell abnormalities such as skewed subgroups. Patients have a propensity for the development of lymphoma, usually in adulthood. At the molecular level, the disorder results from a gain-of-function mutation that leads to constitutive and enhanced activation of the intracellular inflammatory signaling pathway. Treatment with SYK inhibitors rescued human cell abnormalities and resulted in clinical improvement in mice (Wang et al., 2021).
Immunodeficiency 84
MedGen UID:
1794150
Concept ID:
C5561940
Disease or Syndrome
Immunodeficiency-84 (IMD84) is an autosomal dominant primary immunologic disorder characterized by recurrent sinopulmonary infections from childhood associated with low levels of B cells and impaired early B-cell development. There may also be variable T-cell abnormalities. Patients with IMD84 have increased susceptibility to infection with Epstein-Barr virus (EBV) and may develop lymphoma in adulthood (summary by Yamashita et al., 2021).
Immunodeficiency 105
MedGen UID:
1809425
Concept ID:
C5677005
Disease or Syndrome
Immunodeficiency-105 (IMD105) is an autosomal recessive disorder characterized by onset of recurrent infections in early infancy. Manifestations may include pneumonia, dermatitis, and lymphadenopathy. B-cell lymphoma was reported in 1 patient. Laboratory studies show decreased or absent numbers of nonfunctional T cells, normal or increased levels of B cells, hypogammaglobulinemia, and normal or low NK cells. The disorder is caused by a deficiency of transmembrane protein CD45 (PTPRC) on leukocytes, which plays an important role in T- and B-cell development (Cale et al., 1997; Kung et al., 2000). For a general phenotypic description and a discussion of genetic heterogeneity of autosomal recessive SCID, see 601457.
Joint contractures, osteochondromas, and B-cell lymphoma
MedGen UID:
1824078
Concept ID:
C5774305
Disease or Syndrome
Joint contractures, osteochondromas, and B-cell lymphoma (JCOSL) is an autosomal recessive systemic disorder characterized by the development of painless fixed contractures of the joints in early childhood. There is evidence of abnormal chondrocyte homeostasis, resulting in contractures, osteopenia, and the development of osteochondromas. Laboratory studies show abnormal levels and function of B- and T-cell subsets, and patients can develop B-cell lymphomas or malignancies. Despite the abnormalities in immunologic cells, immunodeficiency is not a feature of the disease, suggesting that it can be classified as a 'primary immune regulatory disorder' (Sharma et al., 2022).

Professional guidelines

PubMed

Jacobsen E
Am J Hematol 2022 Dec;97(12):1638-1651. Epub 2022 Oct 18 doi: 10.1002/ajh.26737. PMID: 36255040
Hayden PJ, Roddie C, Bader P, Basak GW, Bonig H, Bonini C, Chabannon C, Ciceri F, Corbacioglu S, Ellard R, Sanchez-Guijo F, Jäger U, Hildebrandt M, Hudecek M, Kersten MJ, Köhl U, Kuball J, Mielke S, Mohty M, Murray J, Nagler A, Rees J, Rioufol C, Saccardi R, Snowden JA, Styczynski J, Subklewe M, Thieblemont C, Topp M, Ispizua ÁU, Chen D, Vrhovac R, Gribben JG, Kröger N, Einsele H, Yakoub-Agha I
Ann Oncol 2022 Mar;33(3):259-275. Epub 2021 Dec 16 doi: 10.1016/j.annonc.2021.12.003. PMID: 34923107
Brudno JN, Kochenderfer JN
Blood Rev 2019 Mar;34:45-55. Epub 2018 Nov 14 doi: 10.1016/j.blre.2018.11.002. PMID: 30528964Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas, 2024

Recent clinical studies

Etiology

Silkenstedt E, Salles G, Campo E, Dreyling M
Lancet 2024 May 4;403(10438):1791-1807. Epub 2024 Apr 10 doi: 10.1016/S0140-6736(23)02705-8. PMID: 38614113
Moleti ML, Testi AM, Foà R
Br J Haematol 2020 Jun;189(5):826-843. Epub 2020 Mar 6 doi: 10.1111/bjh.16461. PMID: 32141616
Ayyappan S, Maddocks K
J Hematol Oncol 2019 Jul 25;12(1):82. doi: 10.1186/s13045-019-0752-3. PMID: 31345247Free PMC Article
Rosenthal A, Younes A
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Ponce RA, Gelzleichter T, Haggerty HG, Heidel S, Holdren MS, Lebrec H, Mellon RD, Pallardy M
J Immunotoxicol 2014 Jan-Mar;11(1):1-12. Epub 2013 Jun 7 doi: 10.3109/1547691X.2013.798388. PMID: 23746314

Diagnosis

Novo M, Castellino A, Nicolosi M, Santambrogio E, Vassallo F, Chiappella A, Vitolo U
Expert Rev Hematol 2019 Jul;12(7):497-506. Epub 2019 Jun 19 doi: 10.1080/17474086.2019.1624157. PMID: 31150587
Kroft SH, Harrington AM
Clin Lab Med 2017 Dec;37(4):697-723. Epub 2017 Aug 31 doi: 10.1016/j.cll.2017.07.001. PMID: 29128065
Solé C, Larrea E, Di Pinto G, Tellaetxe M, Lawrie CH
Cancer Lett 2017 Oct 1;405:79-89. Epub 2017 Jul 27 doi: 10.1016/j.canlet.2017.07.020. PMID: 28757417
Vaqué JP, Martínez N, Batlle-López A, Pérez C, Montes-Moreno S, Sánchez-Beato M, Piris MA
Haematologica 2014 Feb;99(2):222-31. doi: 10.3324/haematol.2013.096248. PMID: 24497559Free PMC Article
Angelopoulou MK, Kalpadakis C, Pangalis GA, Kyrtsonis MC, Vassilakopoulos TP
Leuk Lymphoma 2014 Jun;55(6):1240-50. Epub 2013 Nov 12 doi: 10.3109/10428194.2013.840888. PMID: 24004184

Therapy

Minard-Colin V, Aupérin A, Pillon M, Burke GAA, Barkauskas DA, Wheatley K, Delgado RF, Alexander S, Uyttebroeck A, Bollard CM, Zsiros J, Csoka M, Kazanowska B, Chiang AK, Miles RR, Wotherspoon A, Adamson PC, Vassal G, Patte C, Gross TG; European Intergroup for Childhood Non-Hodgkin Lymphoma; Children’s Oncology Group
N Engl J Med 2020 Jun 4;382(23):2207-2219. doi: 10.1056/NEJMoa1915315. PMID: 32492302Free PMC Article
Burger JA
Cancer J 2019 Nov/Dec;25(6):386-393. doi: 10.1097/PPO.0000000000000412. PMID: 31764119Free PMC Article
Shah NN, Fry TJ
Nat Rev Clin Oncol 2019 Jun;16(6):372-385. doi: 10.1038/s41571-019-0184-6. PMID: 30837712Free PMC Article
Riedell PA, Smith SM
Cancer 2018 Dec 15;124(24):4622-4632. Epub 2018 Sep 25 doi: 10.1002/cncr.31646. PMID: 30252929
Xu-Monette ZY, Zhou J, Young KH
Blood 2018 Jan 4;131(1):68-83. Epub 2017 Nov 8 doi: 10.1182/blood-2017-07-740993. PMID: 29118007Free PMC Article

Prognosis

Cappell KM, Sherry RM, Yang JC, Goff SL, Vanasse DA, McIntyre L, Rosenberg SA, Kochenderfer JN
J Clin Oncol 2020 Nov 10;38(32):3805-3815. Epub 2020 Oct 6 doi: 10.1200/JCO.20.01467. PMID: 33021872Free PMC Article
Paul F, Cartron G
Expert Rev Clin Immunol 2019 Apr;15(4):383-389. Epub 2019 Jan 11 doi: 10.1080/1744666X.2019.1562905. PMID: 30580638
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Blood 2007 Mar 1;109(5):1857-61. Epub 2006 Nov 14 doi: 10.1182/blood-2006-08-038257. PMID: 17105812
Pfreundschuh M, Trümper L, Osterborg A, Pettengell R, Trneny M, Imrie K, Ma D, Gill D, Walewski J, Zinzani PL, Stahel R, Kvaloy S, Shpilberg O, Jaeger U, Hansen M, Lehtinen T, López-Guillermo A, Corrado C, Scheliga A, Milpied N, Mendila M, Rashford M, Kuhnt E, Loeffler M; MabThera International Trial Group
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Rodriguez J, Pugh WC, Cabanillas F
Blood 1993 Sep 1;82(5):1586-9. PMID: 8364208

Clinical prediction guides

Shouval R, Alarcon Tomas A, Fein JA, Flynn JR, Markovits E, Mayer S, Olaide Afuye A, Alperovich A, Anagnostou T, Besser MJ, Batlevi CL, Dahi PB, Devlin SM, Fingrut WB, Giralt SA, Lin RJ, Markel G, Salles G, Sauter CS, Scordo M, Shah GL, Shah N, Scherz-Shouval R, van den Brink M, Perales MA, Palomba ML
J Clin Oncol 2022 Feb 1;40(4):369-381. Epub 2021 Dec 3 doi: 10.1200/JCO.21.02143. PMID: 34860572Free PMC Article
Jacobson CA, Armand P
Best Pract Res Clin Haematol 2018 Sep;31(3):299-305. Epub 2018 Jul 23 doi: 10.1016/j.beha.2018.07.015. PMID: 30213400
Izzo F, Landau DA
Curr Opin Hematol 2016 Jul;23(4):392-401. doi: 10.1097/MOH.0000000000000258. PMID: 27135978
Vaqué JP, Martínez N, Batlle-López A, Pérez C, Montes-Moreno S, Sánchez-Beato M, Piris MA
Haematologica 2014 Feb;99(2):222-31. doi: 10.3324/haematol.2013.096248. PMID: 24497559Free PMC Article
Hans CP, Weisenburger DD, Greiner TC, Gascoyne RD, Delabie J, Ott G, Müller-Hermelink HK, Campo E, Braziel RM, Jaffe ES, Pan Z, Farinha P, Smith LM, Falini B, Banham AH, Rosenwald A, Staudt LM, Connors JM, Armitage JO, Chan WC
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Recent systematic reviews

Ridwansyah H, Wijaya I, Bashari MH, Sundawa Kartamihardja AH, Hernowo BS
Biomol Biomed 2023 Sep 4;23(5):727-739. doi: 10.17305/bb.2023.8791. PMID: 37004241Free PMC Article
Pasqui DM, Latorraca CDOC, Pacheco RL, Riera R
Eur J Haematol 2022 Dec;109(6):601-618. Epub 2022 Sep 18 doi: 10.1111/ejh.13851. PMID: 36018500
Sorigue M, Cañamero E, Miljkovic MD
Blood Rev 2021 May;47:100778. Epub 2020 Nov 8 doi: 10.1016/j.blre.2020.100778. PMID: 33187810
Sokol L, Naghashpour M, Glass LF
Cancer Control 2012 Jul;19(3):236-44. doi: 10.1177/107327481201900308. PMID: 22710899
Gao G, Liang X, Jiang J, Zhou X, Huang R, Chu Z, Zhan Q
Acta Oncol 2010;49(1):3-12. doi: 10.3109/02841860903150502. PMID: 19714523

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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas, 2024

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